Clinical characteristics and outcomes of acquired hemophilia A: experience at a single center in Japan
Clinical characteristics and outcomes of acquired hemophilia A: experience at a single center in Japan
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DOI：
10.1007/s12185-017-2210-8
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发表时间：
2017-07-01
影响因子：
2.1
通讯作者：
Handa, Hiroshi
中科院分区：
文献类型：
--
作者：
Ogawa, Yoshiyuki;Yanagisawa, Kunio;Handa, Hiroshi
获得性血友病A（AHA）由抗凝血因子VIII（FVIII）的自身抗体引起，是一种罕见的、危及生命的出血性疾病，随着人口老龄化，其在日本的发病率似乎在增加。然而，由于这种疾病的罕见性，AHA的临床特征、治疗和结局仍然难以确定。我们回顾性分析了1999年至2015年在群马大学医院诊断为AHA的25例患者（中位年龄73岁;范围24-92岁;男性n = 15）的数据。我们确定自身免疫性疾病和恶性肿瘤的基础条件，分别在四个和三个病人。FVIII抑制剂（中位数47.0 BU/mL;范围2.0-1010）显著降低了所有患者的因子VIII活性（中位数2.0%;范围< 1.0-8.0）。在71例出血事件中，皮下或肌内出血最常见。17例患者需要旁路制剂。24例接受免疫抑制剂治疗的患者中有22例（91.7%）在中位57.5天（范围19-714天）内达到完全缓解（CR）。虽然有3名患者（12%）复发，7名（28%）死于感染，但没有一例死亡与出血有关。虽然我们的大多数患者在免疫抑制治疗后达到CR，但感染相关死亡率仍不令人满意。
Acquired hemophilia A (AHA), which is caused by autoantibodies against coagulation factor VIII (FVIII) is a rare, life-threatening bleeding disorder, the incidence of which appears to be increasing in Japan as the population ages. However, the clinical characteristics, treatment, and outcomes of AHA remain difficult to establish due to the rarity of this disease. We retrospectively analyzed data from 25 patients (median age 73 years; range 24-92 years; male n = 15) diagnosed with AHA between 1999 and 2015 at Gunma University Hospital. We identified autoimmune diseases and malignancy as underlying conditions in four and three patients, respectively. Factor VIII activity was significantly decreased in all patients (median 2.0%; range < 1.0-8.0) by FVIII inhibitor (median 47.0 BU/mL; range 2.0-1010). Among 71 bleeding events, subcutaneous or intramuscular hemorrhage was the most prevalent. Seventeen patients required bypassing agents. Twenty-two (91.7%) of 24 patients treated with immunosuppressive agents achieved complete response (CR) during a median of 57.5 days (range 19-714 days). Although three patients (12%) relapsed and seven (28%) died of infection, none of the deaths were related to bleeding. Although most of our patients achieved CR after immunosuppressive therapy, the rate of infection-related mortality was unsatisfactorily high.